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2018 DEBRA International Congress - One month on

8 October 2018

It was only a month ago when more than 250 delegates and global leading experts gathered together for one purpose – sharing knowledge and best practice in fighting against the painful, skin-blistering condition Epidermolysis Bullosa (EB).

From Sweden and Brazil to Australia and the UK, more than 30 DEBRA groups attended the 2018 DEBRA International Congress held in Zermatt, Switzerland. Representation from such a large number of countries provided DEBRA groups with the unique opportunity to network and communicate best practice advice on a range of challenges many of them face.

To help with sharing knowledge, DEBRA UK provided a flyer to showcase their EB Media Ambassador Programme, which helps connect the UK-based EB Community with available media opportunities – including television appearances, journalist requests and more. Formed in 2017, DEBRA UK now has over 35 active EB Community volunteers who have joined the scheme.

Other DEBRA groups shared how they work directly with their local EB Community, even when their governments have no provision for EB care.

DEBRA Switzerland – provided examples of how they empower and inform patients about navigating the system to access services and support, as patients 20 years and over require private insurance.

DEBRA Singapore and South Asia – relayed that many children with EB are abandoned in East Asia, and told other stories of patients in Cambodia lacking access to dressings and healthcare – often never even receiving a diagnosis – despite being a wealthy nation (highest GDP per capita in the world).

This year’s congress also saw the official launch of the Professor Robin Eady Grants – with DEBRA UK pledging £10,000 per year, there is now more than €30,000 funding available to healthcare and research professionals worldwide for the purpose of specialist EB training.

To help reduce duplication and costs, multiple Clinical Practice Guideline panel meetings took place during the event, including some meetings that focused on developing patient-focused materials so members of the EB Community will be able to better understand the best practice advice. Dr Michael Bärtschi from Switzerland presented information to be included in the guideline mentioning eye care, specifically on the use of therapeutic contact lenses for the treatment of recurrent corneal erosions due to EB.

According to ClinicalTrials.gov, there are over 72 on-going clinical trials targeting symptoms in EB – it’s no wonder why this year’s congress focused heavily on the topic. Updates on some of the latest projects were presented:

Oleogel-S10 (a gel containing an active ingredient derived from Birchbark extract) – being assessed in a Phase III trial for RDEB & JEB by Amryt Pharma. Topline data is expected to be available in 2019.

Diacerein CCP-020 (1% cream) – is in development to help treat EBS. It is believed to block an important inflammatory signalling pathway in EBS and is currently in Phase III trial by Castle Creek.

Losartan – shown to reduce the body’s chemicals that cause scarring and fibrosis, and relieve the symptoms of RDEB. This is currently in the preclinical assessment phase at the University of Frieburg.

PRUSTEM – mesenchymal stromal stem cells have been found to modify inflammation and aid wound healing in RDEB. The cells provide therapeutic effects and although it is known that they disappear, it is not yet understood why or how the therapeutic effects are achieved. Professor John McGrath and his team of researchers at the St John’s Institute of Dermatology are investigating this to better understand and harness these positive outcomes.

LENTICOL-F – combines a gene therapy using a harmless virus with cell therapy to produce infusions of patients’ gene-corrected fibroblast skin cells that are injected into the skin.

GLOVE 1 – indicates that the generation and evaluation of hand therapy devices for EB maintained separation between fingers; reduced the number of dressing changes needed and degradation of the skin; and that patients like and want to use them.

Other projects – botox is being assessed in EBS patients in France; bone marrow transplants have a mortality risk of greater than 25% but have demonstrated that skin impacted by EB can be reduced; and Erythromycin (an antibiotic) is in a Phase III trial to assess efficacy in reducing the number of skin blisters in children with severe EBS-Dowling Meara.

An update on a recently identified EBS gene (KLHL24) informed the audience that symptoms can include alopecia, dotted and linear scarring alike to burns, as well as patients having a high incidence of cardiomyopathy occurring in their teens.

EB care recommendations and techniques vary from person to person; however, there are many methods that have been validated from across the international EB Community. Some key takeaways mentioned throughout the congress include:

Behaviour has an impact on pain perception – distraction, relaxation (breathing exercises) and imagination (positive thinking) are ways to help minimise pain.

Bleach baths have been found to make itching worse. Instead, avoid hot temperatures and direct sun exposure; use air conditioning when possible; try perfume-free products; keep nails short; and administer Gabapentin or Pregabalin just before bedtime.

Plant-based treatments (aromatherapy, tea, topical application or oil drops under the tongue) may help with EB symptoms.

Patients should use round-ended and super fine toothbrushes for small mouth openings. It is recommended patients also train their mouth daily with a special tool to help stretch the muscles to prevent microstomia (closing up of the mouth).

Hydrotherapy is good to help children with EB to keep active.

An app (iscorEB 2.0) is also now available to physicians to help differentiate between severities and subtype based on the input data.

The next DI Congress is taking place in London 19 – 23 January 2020 as the last day of EB World Congress – the first global congress on EB.

Disclaimer: The recommendations in the this news are part of a report on the congress and we advise that you talk to your doctor about suggestions contained. These are not the recommendations of DEBRA.